Grand Rounds “Will I ever see my baby...

Department of Ophthalmology and Visual Sciences

Rick BlodiAugust 2019

Grand Rounds“Will I ever see my baby again?”

CCAcute, painless bilateral vision loss

HPI– 18 yo WF 2 weeks post-partum presented as

transfer to ULH from outside hospital– Woke up at 4:30 AM to breast feed with

normal vision. Went back to sleep– Woke up at 6:30 AM “completely blind”– “Will I ever see my baby again?”

Patient Presentation

Past Ocular Hx: contact lens wearerPast Medical Hx: 2 wks post-partum (uncomplicated, vaginal, epidural)Fam Hx: UnremarkableMeds: NoneAllergies: Nexium, omeprazoleSocial Hx: No smoking, drugs, or alcohol

ROS: +Nausea, vomiting

History (Hx)

OD OS

VA LP LP/HM?

Pupils 4→3mm No rAPD 4→3mm

IOP 16 mmHg 17 mmHg

EOM full full

CVF Unable Unable

External Exam

Anterior Segment ExamPLE OD OS

External/Lids WNL WNL

Conj/Sclera White and quiet White and quiet

Cornea Clear Clear

Ant Chamber Formed Formed

Iris Flat Flat

Lens Clear Clear

Posterior Segment ExamFundus OD OS

Optic NervePink and sharp, without pallor or edema

Pink and sharp, without pallor or edema

Macula WNL WNL

Vessels WNL WNL

Periphery WNL WNL

CT Head from outside hospital - Normal

MRI

• Several episodes of hypertension• Suffered seizure on the night of admission• Received Ativan, Mg, and Keppra• Was 20/20 OU next morning

Hospital Course

• 18 yo WF with acute, bilateral, painless vision loss with subsequent seizure. Benign eye exam. MRI notable for abnormal hyperintensity changes of bilateral occipital and posterior parietal as well as in the cortical area in right hemisphere

• Differential diagnosis:– Eclampsia– Posterior Reversible Encephalopathy Syndrome– Pituitary apoplexy– Central Serous Retinopathy– Venous Sinus Thrombosis– Posterior Circulation Stroke– Intracranial hemorrhage– Infective encephalitis– Autoimmune encephalitis– Toxic/metabolic encephalopathy

Assessment

• Fast onset• Most common symptoms:

– Headache– Altered mental status – Seizures– Visual disturbances

• Associated w HTN,transplantation, eclampsia, others

Posterior Reversible Encephalopathy Syndrome

• Underlying pathology is cerebral edema• Controversial – two theories

1. Severe HTN causes interruption of brain autoregulation2. Systemic inflammatory state causes endothelial

dysfunction

PRES Pathophysiology

• CT can be normal• MRI: T2-hyperintense vasogenic edema of

white matter in cerebral posterior regions• EEG to identify subclinical seizures• LP: r/o infection or subarachnoid hemorrhage

PRES Workup

Catheter Angiography

• Aggressive blood pressure management• Withdrawal of offending drug • Delivery in eclampsia• Antiepileptics for seizures• Ventilation for obtunded patients• Corticosteroids?• Prognosis: typical recovery within 8 days• Recurrence in 4% of pts

PRES Management

Rule out other causes

Venous Sinus Thrombosis

Herpes simplex encephalitis Basilar artery thrombosis

• Blurred vision• Homonymous hemianopsia • Cortical blindness• Visual hallucinations• Photopsias• Fundus and

pupils normal

PRES Visual disturbances

• Fast onset, headaches, seizures, vision changes

• Association with HTN, eclampsia, others• MRI shows bilateral, posterior edema• Treat underlying cause• Broad differential. Important to rule out other

causes• Important for ophthalmologists to be aware of!

Conclusions about PRES

• Hobson EV, Craven I, Blank SC. Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Perit Dial Int. 2012;32(6):590–594. doi:10.3747/pdi.2012.00152

• Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. The Lancet- Neurology.

• Posterior Reversible Encephalopathy Syndrome, Parts 1 and 2: Controversies Surrounding Pathophysiology of Vasogenic Edema. J Neuro Radiology

• http://blog.clinicalmonster.com/2018/05/17/posterior-reversible-encephalopathy-syndrome/

• BCSC Neuro-ophthalmology• The posterior reversible encephalopathy syndrome: what’s

certain, what’s new? Practical Neurology. May 2011

References